-Video-assisted Thorascopic Surgery (VATS)

-standard of care for small to mod PDA

-started 1993

-only 3 short incisions needed- video camera, dissector, and retractor; minimal discomfort bc not rib spreading

-close w a vascular clip

-Robotically Assisted closure of PDA

-since 2002

-but instruments are designed for adults, need large port...

Surgical Results

-Traditional Surgery

-few recent reports

-1994 Mavroudis (Chicago)- ref 28- 1108 pts fr '47-'93 - 98% had lign & division

-no deaths, no recurrence

-recent yrs- 5% needed pRBC transfusion

-LOS <3days in recent yrs

-VATS clipping of PDA

-1997 Laborde - ref 30 - '91-'96- n=332 pts

-5 needed intraop reposition of the clip bc of residual shunt

-only 1 long term residual shunt- small

-1/8% had recurr laryngeal n dysfx- transient in 5 pts, persistent in 1 pt

-mean 20minute operating time

-avg hosp stay 2 days if >6mo

-2002- Nexafatai in Iran ref 32- n=300 mean 6yo, no important xx, no residual PDA shunt

-3 converted to thoracotomy in adults w dialted DA, 2 had recur laryngeal n xx

-ref 33- Wake Forest 1998- good results...

-Surgical closure in Premie infant

-ref 34- VATS for premie 1999- n=34, mean 940gm, 20 pts <1kg- no mortality, no residual shunt, 4 needed conversion to pen thoracotomy, 1 w intracranial hemorrhage at POD2 and one on POD88 bc of multisystem organ failure...

-many still do reg surg

-ref 35 Finland- 101 VLBW premies in 90s- 3% op mortality, overall mortality 10%

Cath Methods

-...ref 39- overall good results...

Cost of Cath vs Surgery

-ref 40-43- mixed results as to who is cheaper

Robot vs VATS for PDA closure

-ref 27- longer op time for robots, 2 robot pts needed re-op for 2nd clip, same LOS, --> authors say similar outcome w robots

AORTOPULMONARY WINDOW

-in the passed, misdiagnosed as PDA, less of an issue now w echo...

Embryology

-incomplete dvpmt of conotruncal septum

-considers it a spectrum w truncus arteriosus

Anatomy

-Richardson classification -ref 44:

-Type 1 - small defect bn AscAo and MPA, juts above sinus of Valsalva

-Type 2 - at post wall of Asc Ao, at origin of RPA

-Type 3 - RPA arises fr R side of the asc Ao, with completely absent AP septum (DDx fr truncus bc there are separate Ao and PA vlvs)

Associated Anomalies

-1/2 of pts have another xx

-inter Ao arch (usually type A), 2nd ASD, PDA, VSD, DORV, TOF, pulm atresia, HLHS, R arch...

Sx

-usually unrestrictive defect--> Sx like large PDA, w hard to distinguish the murmur fr a large PDA

Rx/Interventional Tx

-diuretics.. can't close by cath...

Surgical Indications

-Dx = indication to close -

-if large, pt is at risk for phtn...

-c/i xx = Eisenmengers Sx

Surgical Mgt

-first repaired by Dr Gross in Boston in 1952 (ref 50)

Simple AP Window:

-mediansternotomy

-resect thymus

-harvest pericardial patch

-inspect and confirm Dx at AP window

-ensure 2 semilunar vlvs

-note origins of carotid arteries

-cannulate Asc Ao and RA

-CP Bypass w mod hypothermia; sometimes do circ arrest so you can move Ao cross clamp

-occlude PAs w tourniquet

-Ao cross clamp, cardiplegia to Ao root, remove branch PA tourniquets

-incise into the AP window directly

-confirm location of coronary ostia soon thereafter

-close Ao window w a patch

-close PA window w a patch

-don't cath the vlv leaflets w your sutures!

-de-air heart, and Ao cross clamp released...

AP Window w Interrupted Ao Arch

-usually the RPA is coming of the Ao w near absent AP septum...

-similar Tx as w a truncus

-cannulate Ao at Asc Ao distally

-RA cannula

-CP Bypass, then quickly place tourniquets at branch PAs --> blood can pass thru AP window into the PA then to PDA then to desc Ao

-can divide PDA

-then put a C-clamp on the distal Ao (desc Ao fr the PDA)

-then a longitudinal arteriotomy at underside of Ao Arch, w a clamp across the prox arch--> brain still perfused via the inomm artery; but cool teh pt ...

-complet the anast of Ao arch and desc Ao

-cardioplegia to Ao root

-remove tourniequets fr the branch PAs

-open the Asc Ao via transverse incision at level of the RPA

-baffle within the Ao to direct Q fr mPA to the RPA, across the posterior wall of the Asc Ao

-use a patch to close the Ao anteriorly (at AP window)

-in an infant/small pt, might avoid the intra-aortic baffle bc can --> supraAo vlv stenosis; instead mobilize the RPA, then transect Asc Ao above and below RPA origin and suture the Ao part above and below PA--> tube like extension--> anastomose this to the R side of the MPA at level of teh AP window

-close the PA end of the AP window...

Surgical Results

-ref 53- 2001 study fr '70-90s - n=38, median 5 wks old, 6.6 yr f/u pd

-65% had additional CHD... (TOF, IAA..),

-3 needed reintervention for GA stenosis

-88% 10yr f/u pd

-PA approach had higher risk for reintervention than Ao approach

-ref 54- 2002 study of 40yrs at Chicago- n=22, 4 w IAA, 3 had RPA fr Ao, 3 w VSD

-5 early deaths, 1 late in teh 1st 16 pts, no deaths in the most recent 6 pts w a transAo patch closure

-....

SINUS OF VALSALVA FISTULA

-very rare, espec in West

-often acquired rather than congen- p sinus of Valsalva aneurysm rupture

-80% in one study were at RCA sinus, 20% fr non coroanry sinus

-fistula is Ao to RV in 75%, Ao to RA in 25%

Assoc xx

-47% had assoc xx- 49% of these were VSD, also AR in older pts,

Sx

-unruptured Valsalva sinus usually ASx, negative exam, but might get AR eventually fr distortion...

-rupture often assoc w an acute event - 70% had sudden acute or acute exacerbation of prior Sx- often w exercise or abrupt change in posture

-pathophys similar to PDA or AP window

-L to R shunt --> vol load on the LV, incr in Qp:Qs

-elevated RV P if Q is directly to the RV fr Ao

-lower diastolic pressures systemically w wide pulse pressure

-Sx of L heart failure in most pts, continuous murmur loudest at precordium

-NHx- poor- mean 3.9 yr survival if untreated (ref 60)

Dx

-CXR- incr pulm markings, big L heart

-if Ao-RA fistula--> may have RA enlargement

-dx on echo...

-may need cath to ddx fr a coronary artery fistula

Rx Tx

-diuretics...

Surgical Indications

-the weakness of the sinus of Valsvalva is thought to be progressive--> Dx of a fistula in and of itself is an indication for surgery, within 1-2 weeks of Dx

Surgical Mgt

-first done in 1957...

-goal: close the hole & reinforce the sinus of Valsalva

-median sternotomy

-CP Bypass

-Arterial cannula distally in Asc Ao

-bicaval canulation

-moderate hypothermia

-cardioplegia

-reverse hockey stick incision in Ao, twd non coronary sinus

-oblique RA incision

-ID fistula = parachute structure w a rupture at most distal extremity, usually @ RV, sometimes @RA

-oversew distal end of the fistula, but the main fistula closure is via the Ao

-small patch at fistula- avoid distorting the RCA ostium or Ao vlv leaflets

-Assoc Ao vlv repair

-if signif AR, doa valvuloplasty

Surgical Results

-ref 58 - 1994 study in China- n=154

-79% fistula fr RCA sinus, remainder fr noncoronary sinus

-73% ruptured to RV, rest to RA, except for <1% to LV

-23% had AR

-4.5% operative mortality

-mean 6yr f/u- pre-op AR and worse Sx at surg predicted worse outcome

-ref 65- 2002 study updated experience fr same hospital

-n=67 - most closed at distal end, not at Ao end, valve replaced in 12 pts, w 1 early death and 1 late death; late xx = residual shunt in 2, paravalvar leak in 1, AR in 1

-ref 66- 1999 study at THI, n=64

-61 simple plication, 52 patch repair, 16 Ao vlv replacement; 58% had Ao vlv replacement or repair

-5 hosp deaths, 2 strokes postop

-mean 5 yr f/u pd - 2 reecur fistula

-ref 67- Toronto n=34 pts over 28 yr pd

-10 direct suture closure, 24 patch repair

-5 early recurrence w primary instead of patch repair

-late Ao vlv replace in 6 pts for AR bc of bicuspid Ao vlv in 3 pts, cusp xx in 2 at the affected sinus, Ao root diln in 2 pts

-ref 68- May clinic , n=31 pts fr 1950s-1990s

-risk of recurrence and need for re-operation was lower when an aortotomy, +/- R ventriculotomy was used, as oppose to the R ventriculotomy alone. Good long term survival

AORTOVENTRICULAR TUNNEL

-very rare

-= tunnel connects Ao lumen to a cavity- usually LV, somtimes RV

-unlike a fistula, it is present at birth, Sx by 1yo

Embryo

-? bc abNl differentiation of primordial muscle that initially forms the base of LV and the conotruncus... failure of the outflow cushions to form the sinus of Valsalva, valvar leaflets, and fibrous interleaflet triangles, and abNl separation of distal outflow tract into theAo and PA

-usually fr the R sinus of Valsalva at the ST junction

-usually --> a large tubular or sacular protuberance that is visible on ant side of the Ao root.

Path

--> AR

-systemic steal during diastole --> wide PP

-unlike w PDA/AP window/fistula there is no stel during systole. No impact on pulmonary circulation (bc it goes to LV)

Sx/Dx

-most get CHF by 1yo

-bounding pulses, wide PP

-to and fro murmur w syst and diast thrill

-dx at echo

Rx Tx

-limited

Surgical Indications

-Dx = indication for surgery, even if ASx

Surgical Mgt

-CP Bypass

-RA cannula

-Ao cross clamp

-cardioplegia...

-reverse hocky stick invision twd non cor sinus

-ID coronary ostea

-tunnel is usually to teh L of the RCA os

-close Ao end of the tunnel w a small patch, leave LV end open to allow wash in/out

Surgical Results

-early case series reported 20% mortality (ref 80), but now mortality is low (ref 81)

-early reports--> late AR common, but they were >5yo at repair (ref 82)

-rare in more recent series